Arterial switch operation in a child with commissural malalignment and unusual coronary anatomy.

The arterial switch operation has evolved to become the treatment of choice for transposition of the great arteries and is one of the greatest success stories in congenital heart surgery. The most crucial step of the operation is the coronary artery translocation; therefore, it is of paramount importance for surgeons to know every single detail about the morphology and spatial relationships of the coronary arteries and the roots of the great vessels. However, sometimes the surgeon may face unfavourable scenarios such as major commissural malalignment and anomalous coronary artery patterns and need to be prepared to carry out a successful coronary artery translocation. Herein, we demonstrate that the trapdoor technique is useful for transferring coronary arteries in a neonate with major commissural malalignment and unusual coronary anatomy during the arterial switch operation.

Truncal valve repair in an adolescent with severe annular dilatation.

Approximately 20% of patients with truncus arteriosus might need a truncal valve procedure within 20 years after anatomical repair due to regurgitation. These patients commonly develop valve regurgitation due to root dilatation with a sufficient amount of good quality valvular tissue. Thus, the reduction of the truncal annulus is the single most important factor to achieve durable repair, especially in patients in whom the Ross procedure is not an option.

Aortic valve repair in a low-birth-weight neonate with poor ventricular function: staged tricuspidization of the unicuspid valve.

Congenital severe isolated aortic stenosis in neonates requires prompt intervention because left ventricular function may deteriorate rapidly. Surgical repair of a stenotic aortic valve in neonates allows debridement of fibrous material and precision commissurotomy. As such, the child would end up with a much better-quality aortic valve compared to the uncontrolled tear that results following balloon dilatation. The meticulous debridement and precise commissurotomy are important in achieving long-term durability of the aortic valve repair, with the expectation that the patient may keep the native aortic valve at least into adolescence or adulthood. If feasible, it is important to avoid placement of patches while repairing the valve, so that the growth potential of the cusps is preserved. The valve could be revisited and repaired further, so that, ideally, the native valve could be retained for life, thus avoiding aortic valve replacement entirely. We present a low-birth-weight neonate with a dysplastic unicuspid aortic valve, severe aortic valve stenosis and decreased left ventricular function who underwent repair of the aortic valve.

Single-stage off-pump repair of coarctation of the aorta and ventricular septal defects in children.

OBJECTIVES: The appropriate approach for surgical repair of coarctation of the aorta with a ventricular septal defect (VSD) remains controversial. This study evaluated the outcomes of primary repair of VSDs with periventricular device closure without cardiopulmonary bypass through a left thoracotomy in patients without arch hypoplasia. METHODS: We selected 21 patients aged <1 year, including 7 neonates, who underwent repair of coarctation of the aorta with periventricular device closure of a VSD. RESULTS: The median occluder size was 6 (range, 5-8) mm. The median mechanical ventilation time was 14 (range, 2-68) h, and the median duration of hospital stay was 11 (range, 7-16) days. No reoperations were required to correct VSD shunting, and the median residual shunt size was 1 (range, 1-2) mm. The median follow-up period was 13 (range, 4-31) months. No late deaths were reported, and no haemodynamically significant pressure gradient at the anastomotic site was observed. The median distal aortic arch z-score was 0.39 (range, -0.1-to 0.9). Only 1 patient had a permanent pacemaker implanted towards the end of the follow-up period. CONCLUSIONS: Periventricular device closure can be used safely for closure of VSD in children with coarctation of the aorta without a hypoplastic aortic arch, even in neonates, to reduce the risk of prolonged cardiopulmonary bypass. This hybrid approach can be performed with a low incidence of rhythm disturbances and residual shunting. However, a meticulous assessment of the VSD anatomy is essential to avoid any unfavourable events.

Residual Lesion Diagnostics in Pediatric Postcardiotomy Extracorporeal Membrane Oxygenation and Its Outcomes.

BACKGROUND: To assess the impact of diagnostic procedures in identifying residual lesions during extracorporeal membrane oxygenation (ECMO) on survival after pediatric cardiac surgery. METHODS: Between January 2012 and December 2017, 74 patients required postcardiotomy ECMO. Patients were retrospectively divided into 2 groups: Group I underwent only echocardiography ([echo only] 46 patients, 62.2%) and group II (echo+) underwent additional diagnostic tests (ie, computed tomography [CT] or cardiac catheterization; 28 patients, 37.8%). Propensity score matching was used to balance the 2 groups by baseline characteristics. RESULTS: Two equal groups (28 patients in each group) were formed by propensity score matching. Fourteen (50%) patients in the echo-only group and 20 (71%) patients in the echo+ group were successfully weaned from ECMO (P = .17). Four (14.3%) patients survived in the echo-only group and 15 (53.5%) patients survived in the echo+ group (P = .004). Patients in the echo+ group had a lower chance of dying compared to the echo-only group (odds ratio, 0.14.6; 95% CI, 0.039-0.52; P = .003). The residual lesions, which may have served as a mortality factor, were found by autopsy in 8 (40%) patients in the echo-only group, while none were found in the echo+ group (P = .014). CONCLUSIONS: The autopsies of patients who died despite postcardiotomy ECMO support showed that in 40% of cases that had been investigated by echo only, residual lesions that had not been detected by echocardiography were present. The cardiac catheterization and CT during ECMO are effective and safe for identifying residual lesions. Early detection and repair of residual lesions may increase the survival rate of pediatric cardiac patients on ECMO.

Outcomes of Double-patch and Warden Techniques in Patients With Supracardiac Partial Anomalous Pulmonary Venous Connection.

AIM: This study compared outcomes following the double-patch and Warden procedures for correcting partial anomalous connection of the right pulmonary veins to the superior vena cava. METHODS: Eighty (80) patients, aged <18years old, and with partial anomalous connection of the right pulmonary veins to the superior vena cava were randomly assigned into double-patch method (n=40) and Warden procedure (n=40) groups. The median follow-up was 22.5 (range, 12-39) months. The primary endpoint was sinus node dysfunction at the mid-term follow-up period. RESULTS: No early or late mortality occurred. In the early postoperative period, sinus node dysfunction was observed in 27.5% and 5% of cases after double-patch correction and the Warden procedure, respectively (risk ratio, 5.50; 95% confidence interval, 1.30-23.25; p=0.01). At follow-up, sinus node dysfunction persisted in two (5%) patients after double-patch correction. All patients had normal sinus rhythm after the Warden procedure. No early or late pacemaker implantation occurred in either group. No patients had significant pulmonary veins or superior vena cava stenosis. CONCLUSIONS: The double-patch technique and Warden procedure both showed excellent early and mid-term results with no mortality and minimal morbidity. The Warden procedure was associated with less sinus node dysfunction in the early postoperative period than the double-patch technique. There was no significant between-group difference in sinus node dysfunction at the mid-term follow-up.

Diepoxy- Versus Glutaraldehyde-Treated Xenografts: Outcomes of Right Ventricular Outflow Tract Reconstruction in Children.

BACKGROUND: Xenografts used for right ventricular outflow tract (RVOT) reconstruction are typically treated with glutaraldehyde. However, potential benefit of epoxy treatment was demonstrated in experimental studies. We aimed to compare diepoxy-treated bovine pericardial valved conduits (DE-PVCs) and glutaraldehyde-treated bovine pericardial valved conduits (GA-PVCs) for RVOT reconstruction in pediatric patients. METHODS: Between 2002 and 2017, 117 patients underwent RVOT reconstruction with PVC in single center: DE-PVC group, n = 39; and GA-PVC group, n = 78. After performing propensity score analysis (1:1) for the entire sample, 29 patients from the DE-PVC group were matched with 29 patients from the GA-PVC group. RESULTS: There were no conduit-related deaths. In the DE-PVC group, the freedom from conduit failure was 90.9% at four years and 54.3% at eight years postoperatively. In the GA-PVC group, it was 46.3% and 33.1%, respectively. The difference was significant (P = .037). Conduit failure was typically caused by stenosis in both groups. In the DE-PVC group, the main cause of stenosis was xenograft calcification (27.6%); while in the GA-PVC group, it was mostly due to neointimal proliferation (25.0%) and, less often, calcification (14.3%). Conduit thrombosis was the cause of replacement in 6.9% of patients from the GA-PVC group. CONCLUSIONS: Diepoxy-treated bovine pericardial valved conduit is a suitable alternative to GA-PVC for RVOT reconstruction in pediatric patients. Diepoxy-treated bovine pericardial valved conduits may be less prone to conduit failure and more resistant to neointimal proliferation and conduit thrombosis than GA-PVCs.

Selective Antegrade Cerebral Perfusion With or Without Additional Lower Body Perfusion During Aortic Arch Reconstruction in Infants.

BACKGROUND: Aortic arch reconstruction is often challenging, especially in infants, owing to its high postoperative complication risks. This study aimed to compare the effectiveness between selective antegrade cerebral perfusion (SACP) alone and SACP in combination with continuous lower body perfusion with descending aortic cannulation (DAC) in preserving renal function, and to determine the influence of perfusion strategy on the postoperative course of infants who underwent aortic arch reconstruction. MATERIAL AND METHODS: A total of 121 infants who underwent aortic arch reconstruction between January 2008 and December 2018 were included in the analysis. Patients (median age: 29 days, range: 3-270 days) were divided into the following groups: those who underwent repair with SACP (SACP group, 79 patients) and those who underwent additional lower body perfusion (DAC group, 42 patients). RESULTS: Three (7.1%) and nine (11.4%) patients died in the DAC and SACP groups, respectively (P = .54). The SACP group had more patients requiring renal replacement therapy (P = .002) and higher incidence of second stage acute kidney injury (AKI) development (Kidney disease improving global outcomes (KDIGO) criteria; P = .032). The SACP group had higher frequency of open chest postoperatively than the DAC group (P = .011). The DAC group had lower vasoactive inotropic score (VIS) at the first postoperative day (P < .001) and shorter intensive care unit length of stay (P = .050). There was no difference in neurological complications between the groups (P = .061). High VIS was associated with early mortality (odds ratio [OR]: 1.79 [1.33-2.41], P < .001) and AKI (OR: 1.60 [1.35-1.91], P < .001). The DAC perfusion strategy with minimal hypothermia was associated with lower risk of AKI (OR: 0.91 [0.84-0.98], P = .016). CONCLUSION: Antegrade cerebral perfusion with continuous lower body perfusion via DAC could effectively be used for improving early postoperative results among infants undergoing procedures that include aortic arch reconstruction.

Vacuum-assisted closure versus closed irrigation for deep sternal wound infection treatment in infants: a propensity score-matched study.

OBJECTIVES: This study aimed to compare vacuum-assisted closure therapy (VAC) and closed irrigation drainage therapy (CID) for deep sternal wound infection treatment in infants. METHODS: From January 2008 to March 2018, 69 patients (1.73%) had deep sternal wound infection. They were divided into 2 groups: patients treated with VAC therapy (VAC group, 29 patients) and those treated with CID therapy (CID group, 40 patients). After performing a propensity score analysis (1:1) for the entire sample, 16 patients receiving VAC therapy were matched with 16 patients receiving CID therapy. RESULTS: No significant difference was noted between both groups regarding age [d = 0.045; 95% confidence interval (CI) 0.99-1.07], gender (d = 0.001; 95% CI 0.22-4.45), weight (d = 0.011; 95% CI 0.73-1.35), body surface area (d = -0.023; 95% CI 0.01-5733.08), cardiopulmonary bypass (d = 0; 95% CI 0.16-5.90) and open chest duration (d = -0.112; 95% CI 0.31-5.16). Five patients died in the CID group (31.25%) during hospital stay, and there were no deaths in the VAC group (P = 0.024). Recurrence of mediastinitis occurred in 1 patient (6.25%) from the VAC group and in 6 patients (37.5%) from the CID group (P = 0.037). The multivariable regression analysis revealed that the CID method was the only risk factor for remediastinitis (odds ratio 17.3; 95% CI 1.04-286.75; P = 0.046). CONCLUSIONS: Use of VAC therapy in infants with deep sternal wound infection was associated with a substantial decrease in the mortality rate and duration of therapy compared with CID technique. The CID technique was an independent risk factor for recurrence of mediastinitis.

Double Arterial Cannulation in the Critical Management of Neonatal Aortic Arch Obstruction With Closed Ductus Arteriosus.

The neonatal management of critical aortic arch obstruction in association with an arterial duct that remains closed or significantly constricted requires urgent surgical repair, with a postoperative focus on resolving the impaired function of compromised organs. Here, we present two neonatal cases illustrating the use of the dual arterial cannulation perfusion method in the complete repair of interrupted aortic arch and critical coarctation of the aorta with nonfunctional ductus arteriosus. We suggest that dual arterial cannulation, which provides total body perfusion (with the exception of cardiac perfusion during cardioplegic arrest of the heart), may be a good alternative to other perfusion strategies in cases of absent ductal blood flow, as this method allowed us to avoid the further development of severe multiple organ failure.

Extracorporeal cardiopulmonary resuscitation after pulmonary artery rupture.

Pulmonary artery rupture during pulmonary balloon valvuloplasty is a rare and life-threatening complication. Here, we present a pulmonary artery rupture in a 10-month-old infant. The patient had a tamponade, ineffective cardiac massage for 40 minutes and extreme hemodilution due to blood loss. Extracorporeal life support was used for three days. The patient was discharged without any neurological sequelae.

In search of the best xenogeneic material for a paediatric conduit: an analysis of clinical data.

OBJECTIVES: In this study, we aimed to determine the incidence of reintervention and calcification of xenografts in paediatric patients who underwent placement of the right ventricle-to-pulmonary artery valved conduits. METHODS: We retrospectively analysed clinical data of paediatric patients (1 day-18 years) who underwent right ventricular outflow tract reconstruction using xenograft from 2000 to 2016 at a single centre. RESULTS: A total of 301 patients underwent the placement of 337 xenografts, including glutaraldehyde-treated bovine jugular vein (n = 171, 50.7%), glutaraldehyde-treated bovine pericardial valved conduit (n = 75, 22.3%), diepoxy-treated porcine aortic conduit (n = 58, 17.2%) and diepoxy-treated bovine pericardial valved conduit (DE-PVC) (n = 33, 9.8%). There were 284 (84.3%) primary implantations and 53 (15.7%) reimplantations. The median follow-up was 4.2 years (range 1.5 months-14.5 years). The multivariate regression analysis did not reveal statistically significant associations of the first reintervention with the type of xenograft (P = 0.78). At reintervention, calcification of the wall and/or cusps was the main cause of conduit dysfunction in 66.4% of cases. On the basis of the multivariate Cox regression analysis, xenograft types were significant predictors of reintervention caused by conduit calcification (P = 0.012). The diepoxy-treated porcine aortic conduit group had the risk of calcification 3 times higher than the glutaraldehyde-treated bovine jugular vein group (P < 0.001).The glutaraldehyde-treated bovine pericardial valved conduit and diepoxy-treated bovine pericardial valved conduit groups had the risk of calcification comparable with the glutaraldehyde-treated bovine jugular vein group in multivariate proportional hazards model (P = 0.36 and P = 0.59, respectively). CONCLUSIONS: We have not revealed significant difference in the freedom from first reintervention among types of conduit. Calcification leading to the conduit dysfunction was present in all groups; however, diepoxy-treated porcine aortic conduits demonstrated suboptimal results in terms of calcification at follow-up.

Influence of staged repair and primary repair on outcomes in patients with complete atrioventricular septal defect and tetralogy of Fallot: a systematic review and meta-analysis.

OBJECTIVES: Complete atrioventricular septal defect with tetralogy of Fallot is a rare congenital heart disease. The combination of these lesions occurs in about 1% of all patients with atrioventricular canal defects and in 5-6% of patients with tetralogy of Fallot. To assess the influence of surgical strategy on the survival and reintervention rate for the left atrioventricular valve and right ventricular outflow tract. METHODS: We analyzed all related studies since 1986. Thirty-eight articles were initially retrieved via PubMed/MEDLINE, Cochrane Central Register of Controlled Trials and Google Scholar, from which 18 retrospective studies were included in the systematic review and 8 studies in the meta-analysis. RESULTS: There was no significant difference in the 6-year survival between staged repair and primary repair [80 patients in the primary group and 81 patients in the staged group; I2 = 0%; time-to-event data Peto odds ratio 0.66, 95% confidence interval (CI) 0.3-1.5, P = 0.31; hazard ratio 0.66, 95% CI 0.3-1.3, P = 0.23]. Both groups had an equal reintervention rate for the left atrioventricular valve [75 patients in the primary group and 71 patients in the staged group; I2 = 0.26%; the Mantel-Haenszel odds ratio 0.60, 95% CI 0.22-1.68, P = 0.33], but patients who received an initial palliation had a higher rate of reoperation on the right ventricular outflow tract [I2 = 0%; the Mantel-Haenszel odds ratio 0.27, 95% CI 0.27-0.9988, P = 0.05]. CONCLUSIONS: Results of this meta-analysis reveal no difference in 6-year survival and reoperation rate for the left atrioventricular valve; however, patients who underwent staged repair had a higher rate of reintervention for the right ventricular outflow tract, which could be related to initially poor pulmonary bed anatomy. Therefore, both the primary repair and the staged repair are acceptable options for repair of complete atrioventricular septal defect with tetralogy of Fallot. The choice of surgical strategy must consider the anatomy of the pulmonary bed, patient condition and associated anomalies, which could affect surgical risk.

Does mitral valve repair matter in infants with ventricular septal defect combined with mitral regurgitation?

OBJECTIVES: This study aimed to assess mitral valve function after repair of ventricular septal defect (VSD) combined with mitral regurgitation (MR) in the mid-term follow-up period, to evaluate the clinical utility of simultaneous mitral valve repair (MVR). METHODS: From June 2005 to March 2014, 60 patients with VSD and MR underwent surgical treatment. After performing propensity score analysis (1:1) for the entire sample, 46 patients were selected and divided into 2 groups: those with VSD closure and MVR (VSD + MVR, 23 patients) and those with VSD closure without mitral valve intervention (VSD only, 23 patients). The follow-up period ranged from 12 to 48 months (median 32 months; interquartile range 28-40 months). RESULTS: There was no postoperative mortality in either group. There was no significant difference in the duration of the postoperative period between groups (ventilation time, P = 0.49; inotropic support, P = 0.50). Mean cardiopulmonary bypass time and aortic cross-clamping time were significantly longer in the 'VSD + MVR' group (cardiopulmonary bypass, P = 0.023; aortic cross-clamp, P < 0.001). There was no significant difference in regurgitation area (P = 0.30) and MR grade (P = 0.76) between groups postoperatively. There was no significant difference in freedom from MR ≥ 2+ between groups (log-rank test, P = 0.28). In the 'VSD + MVR' group, 12-, 36- and 42-month freedom from MR ≥ 2+ values were 100%, 59 ± 10.4% [95% confidence interval (CI) 36.1-76.2%] and 44 ± 15% (95% CI 15.8-69.7%), respectively, while in the 'VSD only' group, these values were 100%, 54.5 ± 10.6% (95% CI 32-72.3%) and 54.5 ± 10.6% (95% CI 30-72.3%), respectively. The only significant risk factor for recurrent MR ≥ 2+ during the follow-up period was mild residual MR in the early postoperative period (P = 0.037). CONCLUSIONS: In infants with VSD combined with MR, simultaneous MVR has no benefits simultaneous MVR provided no advantage over that of isolated VSD closure. We found that the presence of mild residual MR in the early postoperative period predisposes the development of MR ≥ 2+ in follow-up period.

Postcardiotomy refractory ventricular fibrillation: rescue using veno-arterial extracorporeal membrane oxygenation.

We present a case of 7-hour ventricular fibrillation with successful use of veno-arterial extracorporeal membrane oxygenation as a bridge to recovery in a 30-year-old patient with grown-up congenital heart disease who underwent pulmonary valve replacement.

The Warden procedure can be successfully performed using minimally invasive cardiac surgery without aortic clamping.

Currently, minimally invasive cardiac surgery has found widespread use even in congenital heart surgery. The number of defects, which can be corrected through a small incision or totally endoscopic, is on the rise. Nowadays, surgeons can repair atrial septal defect, ventricular septal defect, patent ductus arteriosus and other congenital heart defects using minimally invasive techniques. In this paper, we report 21 cases of successful repair of supracardiac partial anomalous right upper and middle pulmonary venous connection, using the Warden procedure. It was performed in children through the right-sided midaxillary thoracotomy with direct cardiopulmonary bypass cannulation and induction of ventricular fibrillation. There were no operative or early postoperative deaths or complications. All patients were in sinus rhythm at discharge. According to echocardiography, there were no cases of early SVC or pulmonary veins narrowing. The Warden procedure can be performed safely and efficiently using the minimally invasive cardiac surgery.